Coved-type ST-segment Elevation Research Articles

Lower 24-hour Holter-Derived Heart Rates Suggest Brugada Syndrome: Study of the Family

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

The changes of QT interval in standing position – new criteria for diagnostic syndrome (LQTS)

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

Mutational Spectrum of the SCN5A gene in Brugada Syndrome Patients

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

Arrhythmogenecity of Right Ventricular Outflow Tract Epicardium -A Simulation Study

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

Permanent His-Bundle Pacing instead of Biventricular Pacing for Cardiac Resynchronization Therapy: a Case Report

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

Type 1 Brugada phenocopy in a patient with acute pericarditis

We herein describe a case of an acute pericarditis, in which type 1 Brugada phenocopy (BrP) was documented. The patient was referred to our hospital due to severe chest pain. The twelve-lead electrocardiogram (ECG) on admission showed type 1 Brugada ECG pattern (coved-type) in the precordial leads. Echocardiography only showed mild pericardial effusion. However, his ST segment elevation returned to normal and chest discomfort disappeared 3 weeks later. Our report addresses the possibility that the coved-type ST-segment elevation cannot be a sensitive finding for Brugada syndrome (BrS). Detailed tests are anyway needed to make appropriate diagnostic and therapeutic decisions.

Multiple serial ECGs aid with the diagnosis and prognosis of Brugada syndrome

BackgroundA spontaneous coved-type ST segment elevation in the electrocardiogram (ECG) has long been recognized as a risk stratification tool in patients with Brugada syndrome (BrS). This Type-I ST segment elevation is known to exhibit high dynamicity, fluctuating between coved-type and non-coved ST segment elevation. Our objectives in this study were to: 1) Compare ECG parameters in patients with spontaneous coved-type (Type-I) vs. non-coved-type ST segment ECGs; 2) Determine the variability of these ECG parameters with repeated measurements; and 3) Assess the predictive value of ECG parameters in these two groups during follow-up. MethodsForty-two consecutive patients with BrS and implanted ICD were studied between 2000 and 2017. Serial ECGs and clinical characteristics were obtained over a period of 199 months. ResultsQT-interval, QTc-interval, QRS duration, Tp-e interval and Tp-e dispersion were all significantly longer in spontaneous Type I vs. non-Type 1 ECGs and all ECG parameters displayed significant variability during serial recording obtained throughout the follow-up period. Patients with a spontaneous Type I ECG during the 114 ± 56 months follow-up period were at a much higher risk for VT/VF than those without a Type I ECG (p = 0.016). Moreover, the risk for development of life-threatening ventricular arrhythmias was directly related to the fraction of ECGs displaying a spontaneous Type I pattern during follow-up. ConclusionOur study illustrates the need for multiple ECGs to aid with both the diagnosis and prognosis of BrS. Serial ECGs can assist with risk stratification based on the fraction of ECGs that display a spontaneous Type-I BrS ECG.

Calcium in Brugada Syndrome: Questions for Future Research.

The Brugada syndrome (BrS) is characterized by coved-type ST-segment elevation in the right precordial leads on the electrocardiogram (ECG) and increased risk of sudden cardiac death (SCD). While it is an inheritable disease, determining the true prevalence is a challenge, since patients may report no known family history of the syndrome, present with a normal spontaneous ECG pattern at the time of examination, and test negative for all known BrS-causative genes. In fact, SCD is often the first indication that a person is affected by the syndrome. Men are more likely to be symptomatic than women. Abnormal, low-voltage, fractionated electrograms have been found in the epicardium of the right ventricular outflow tract (RVOT). Ablation of this area abolishes the abnormal electrograms and helps to prevent arrhythmic recurrences. BrS patients are more likely to experience ventricular tachycardia/fibrillation (VT/VF) during fever or during an increase in vagal tone. Isoproterenol helps to reverse the ECG BrS phenotype. In this review, we discuss roles of calcium in various conditions that are relevant to BrS, such as changes in temperature, heart rate, and vagal tone, and the effects of gender and isoproterenol on calcium handling. Studies are warranted to further investigate these mechanisms in models of BrS.

The definition of the Brugada syndrome

Brugada syndrome (BS) is an inherited disease characterized by a coved-type ST-segment elevation in the right precordial leads and increased risk of sudden cardiac death (SCD), in the absence of structural abnormalities. The cornerstone of BS diagnosis and definition, is its characteristic ECG pattern that can be present spontaneously or unmasked by drugs. Brugada syndrome was first described 25 years ago; paradoxically, in an era of great technological development, a new syndrome was described with a technology developed almost a century before. Great scientific knowledge has been gathered since the description of the syndrome. The better understanding of its pathophysiology and genetic basis has led to several modifications in its definition. Despite these facts, the essential, the description of the specific ECG pattern has remained almost unchanged since the initial report. In this article, we present the definition of the BS, the rationale behind it and our thoughts about its future.

Optimal management of Brugada syndrome

Brugada syndrome can induce malignant ventricular arrhythmias in the absence of structural heart disease. The hallmark of the condition is the ’’coved’’ (type I) or ’’saddle-back’’ (types II and III) ST segment elevation in the right precordial leads. The definite diagnosis requires the documentation of the type I morphology. The electrocardiographic pattern is often dynamic, but it can be unmasked by sodium channel blockers such as flecainide. We report the case of a 33-year old male, with family history significant for sudden cardiac death, who underwent successful cardio-pulmonary resuscitation for ventricular fibrillation-associated cardiac arrest. The 12 lead electrocardiogram performed after the resuscitation maneuvers showed intermittent type 1 Brugada pattern. General physical examination and routine laboratory evaluation were unremarkable. A repeated electrocardiogram revealed sinus tachycardia with right bundle branch block. We performed a flecainide challenge test which reproduced the initial coved-type ST segment elevation in V1 and V2. We decided to implant a ventricular single chamber cardioverter-defibrillator with one therapeutical window for ventricular fibrillation (at 300 ms), high energy shocks without antitachycardia pacing. The device was successful in preventing another episode of ventricular fibrillation just 2 days after the procedure. Implantable cardioverter-defibrillators are the most effective secondary prophylaxis therapeutic options for individuals with Brugada Syndrome, but they subject the patient to complications related to device implantation and inappropriate shocks.

Fever induced Brugada syndrome masquerading as STEMI

Brugada Syndrome (BrS) is an autosomal dominant genetic disease determined by abnormal electrocardiographic (ECG) pattern and causes increased risk of sudden cardiac death. It is characterised by coved-type ST segment elevation in precordial leads V1-V3. There had been many proposed mechanisms explaining the link of sodium channel mutations and its electrophysiology. There are various triggering factors like fever, hypokalemia, ischemia, cocaine abuse and medications which unmask the ECG changes and these changes are usually transient. There is increased possibility of this cardiac rhythm to deteriorate into life threatening arrhythmias. Thus it is essential for the clinician to be aware of the conditions. We present such a scenario in which hyperthermia unmasked our patient’s Brugada syndrome.

47. A cardiac center experience with Brugada syndrome who survived sudden cardiac death

Brugada syndrome is a heritable arrhythmia syndrome that is characterized by an electrocardiographic pattern consisting of coved-type ST-segment elevation (2 mm) followed by a negative T wave in the right precordial leads, V1 through V3 (often referred to as type 1 Brugada electrocardiographic pattern), here we describe 3 cases of Brugada who survived sudden cardiac death (SCD) cardiac center experience with survived Brugada syndrome patients – case series. First Case: The Father 45 years old male, presented in 2005 after involvement in unprovoked motor vehicle accident, the patient was the driver who lost consciousness and rushed to the hospital. On arrival to our ER and putting the patient on the bed, the ER doctor observed a brief episode of VF on the monitor. The patient was taken to the catheterization Lab , his coronaries were normal. The diagnosis of Brugada was established and the patient received a defibrillator. At That Time all family members were screened and were negative. Second Case: The Son of the first patient 5 years later his 23 years old male rushed to our ER after he lost consciousness, he was passenger in the car of his friend. Third Case: The pilot A military pilot aged a male 35 years old was in very good health when he lost consciousness and brought to the hospital after resuscitation in 2005. He had full invasive cardiac evaluation, subsequently he received a defibrillator in the same admission period, till 2015 he is doing fine. Brugada syndrome is associated with high tendency for sudden cardiac death. In our three cases the first clinical presentation was survived sudden cardiac death (SCD) and all three male patients survived. We did not encounter a female patient who survived sudden cardiac death.

Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management.

Brugada syndrome is an inherited arrhythmogenic disorder, characterised by coved-type ST-segment elevation in the right precordial leads, and is associated with increased risk of sudden death. It is genetically and clinically heterogeneous, presenting typically in the fourth or fifth decade of life. The prevalence of Brugada syndrome in the paediatric population is low compared with the adult population. Interestingly, over the last several years, there has been growing evidence in the literature of onset of the disease during childhood. Most of the paediatric cases reported in the literature consist of asymptomatic Brugada syndrome; however, some patients manifest the disease at different regions of the cardiac conduction system at a young age. Early expression of the disease can be affected by multiple factors, including genetic substrate, hormonal changes, and still unknown environmental exposures. The initial manifestation of Brugada syndrome in children can include sinus node dysfunction and atrial arrhythmias. Brugada syndrome can also manifest as ventricular arrhythmias leading to sudden death at an early age. In symptomatic children, performance of the ajmaline test by an experienced team can be safely used as a diagnostic tool to unmask latent Brugada syndrome. Defining indications for an implantable cardioverter defibrillator in children with the diagnosis of Brugada syndrome remains challenging. Given the rarity of the syndrome in children, most paediatric cardiologists will only rarely see a young patient with Brugada syndrome and there is still no universal consensus regarding the optimal management approach. Care should be individualised according to the specific clinical presentation, taking into account the family history, genetic data, and the family's specific preferences.

Low amplitude ECG and QRS fragmentation in provocable coved-type ST-segment elevation on surface ECG are strong predictors of a continuum between arrhythmogenic cardiomypathy and Brugada syndrome

It has been long discussed whether arrhythmogenic cardiomyopathy and Brugada syndrome have a connection to each other. Since the publication of the so-called connexome as an association of desmosomal mutations, sodium channel complexes and gap junctions this concept is more and more evident [ 1 Ten Sande J.N. Coronel R. Conrath C.E. Driessen A.H. de Groot J.R. Tan H.L. et al. ST-segment elevation and fractionated Electrograms in Brugada syndrome patients arise from the same structurally abnormal subepicardial RVOT area but have a different mechanism. Circ. Arrhythm. Electrophysiol. 2015; 8: 1382-1392 PubMed Google Scholar , 2 Bayes de Luna A. Garcia-Niebla J. Baranchuk A. New electrocardiographic features in Brugada syndrome. Curr. Cardiol. Rev. 2014; 10: 175-180 Crossref PubMed Google Scholar ]. A continuum between arrhythmogenic cardiomyopathy and Brugada syndrome is discussed.

A Computational Modeling Investigation into SCN10A Linked Brugada Syndrome

A functional role for the sodium channel NaV1.8 in the heart is unclear. Mutations and polymorphisms in SCN10A, the gene encoding NaV1.8, have recently been linked to Brugada Syndrome, an inherited cardiac disease that causes sudden death. The Brugada Syndrome is detected on an ECG by the hallmark appearance of coved type ST-segment elevation (J-wave) and is associated with a loss of NaV channel function in the heart. While wild-type NaV1.8 has been shown in recent studies to modify and promote NaV1.5 mediated current, two mutations in the SCN10A gene, R14L and R1268Q, result in a loss of cardiac NaV function. We utilized a computational modeling and simulation approach to predict if mutation induced loss of function in the cardiac INa by R14L and R1268Q is a plausible mechanism of the Brugada Syndrome phenotype. We have developed models of the R14L and R1268Q NaV1.8 mutations and their interactions with the canonical cardiac Na channel, NaV1.5, based on published experimental data. We then used these models to predict the effect of the mutations on cardiac cellular excitability and computed electrograms from simulated tissue. The model predicts that in the single cell, both two mutations result in major depression of cellular excitability, but action potentials were successfully generated throughout the range of physiological pacing frequencies. In the tissue level simulations, the predictions were very different: Simulated tissue homozygous for either mutation resulted in complete failure of propagation. When we tested the effect of the heterozygous (50%) mutant effect, the simulation predicted the hallmark appearance of coved type ST-segment elevation (J-wave). Our results suggest that the defects in the SCN10A gene that modify INa in the heart can explain the clinically observed phenotype in patients.

The correlation between epsilon wave and coved-type ST-segment elevation in a patient with arrhythmogenic cardiomyopathy

A 53-year female patient was successfully resusicated from electrical storm containing polymorphic ventricular tachycardia and recurrent ventricular fibrillation as the first manifestation of cardiac disease.

An overlap of Brugada syndrome and arrhythmogenic right ventricular cardiomyopathy/dysplasia

Overlapping characteristics of Brugada syndrome (BrS) and arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) have been reported in recent studies, but little is known about the overlapping disease state of BrS and ARVC/D. A 36-year-old man, hospitalized at our institution for syncope, presented with this overlapping disease state. The electrocardiogram showed spontaneous coved-type ST-segment elevation, and ventricular fibrillation was induced by right ventricular outflow tract stimulation in an electrophysiological study. BrS was subsequently diagnosed; additionally, the presence of epsilon-like waves and right ventricular structural abnormalities met with the 2010 revised task force criteria for ARVC/D. After careful investigation for both BrS and ARVC/D, an implantable cardioverter defibrillator was inserted in the patient. This case revealed 2 important clinical findings: (1) BrS and ARVC/D clinical features can coexist in a single patient, and EPS might be useful for determining the phenotype of overlapping disease (e.g., BrS-like or ARVC/D-like). (2) An overlapping disease state of BrS and ARVC/D can change phenotypically during its clinical course. Therefore, careful examination and attentive follow-up are required for patients with BrS or ARVC/D.

A viewpoint on monitoring strategies in a patient with Bruguda Syndrome. A commentary by Dr Andreas Vogt

Brugada Syndrome was first described in 1992 in eight patients showing recurrent episodes of aborted sudden cardiac death [1,2]. Typically the hearts of affected patients did not show any structural disease, however, a common sign of the syndromewas right bundle branch block with a coved type ST-segment elevation in the precordial electrocardiogram (ECG) leads (type I Brugada ECG) [3,4]. Mutations of key cardiac ion channels have been found responsible for a spectrum of several heritable cardiac arrhythmia syndromes like Brugada syndrome, long QT-syndrome (LQTS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) [5]. Around 300 pathogenic variations in 16 genes are attributed to the channelopathies of Brugada syndrome. A pathology of the SCN5A gene can be found in 25%e30% of diagnosed patients [4,6]. However, recent findings questioned the concept of Brugada syndrome as a monogenic disease, and suggested a key role for three genes: SCN10A, SCN5A and HEY2 [7,8]. In Europe the prevalence was reported 1 to 5 of 10,000, however, in Southeast Asia the prevalence is more than double with a men towomen ration of 8:1 [6,9]. Due to this significant prevalence in Asia, Brugada syndrome might be the main reason for natural death in Asian men younger than 50 years [4]. In 2011 the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA) consensus statement on the state of genetic testing for the channelopathies and cardiomyophathies recommended genetic testing for Brugada syndrome as follows:

Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects

Brugada syndrome (BrS) is an inherited cardiac arrhythmia syndrome first described as a new clinical entity in 1992. Electrocardiographically characterized by distinct coved type ST segment elevation in the right-precordial leads, the syndrome is associated with a high risk for sudden cardiac death in young adults, and less frequently in infants and children. The electrocardiographic manifestations of BrS are often concealed and may be unmasked or aggravated by sodium channel blockers, a febrile state, vagotonic agents, as well as by tricyclic and tetracyclic antidepressants. An implantable cardioverter defibrillator is the most widely accepted approach to therapy. Pharmacologic therapy is designed to produce an inward shift in the balance of currents active during the early phases of the right ventricular action potential (AP) and can be used to abort electrical storms or as an adjunct or alternative to device therapy when use of an implantable cardioverter defibrillator is not possible. Isoproterenol, cilostazol, and milrinone boost calcium channel current and drugs like quinidine, bepridil, and the Chinese herb extract Wenxin Keli inhibit the transient outward current, acting to diminish the AP notch and thus to suppress the substrate and trigger for ventricular tachycardia or fibrillation. Radiofrequency ablation of the right ventricular outflow tract epicardium of patients with BrS has recently been shown to reduce arrhythmia vulnerability and the electrocardiographic manifestation of the disease, presumably by destroying the cells with more prominent AP notch. This review provides an overview of the clinical, genetic, molecular, and cellular aspects of BrS as well as the approach to therapy.

Is Brugada syndrome a variant of arrhythmogenic cardiomyopathy?

Brugada syndrome is characterized by coved-type ST-segment elevation in right precordial leads after exclusion of structural heart disease.